60 let Ústavu hematologie a krevní transfuze Praha

M. Trněný

Vnitr Lek 2012, 58(Suppl 2):7  

Myelodysplastic syndromes. Progress in diagnosis and treatment within 30 years of myelodysplastic syndromes registry in Institute of Hematology in Prague

J. Čermák

Vnitr Lek 2012, 58(Suppl 2):8-15  

An analysis of data obtained from 367 patients with primary myelodysplastic syndromes (MDS) treated in Institute of Hematology and Blood Transfusion in the years 1980-2009 revealed substantional changes in diagnostic and therapeutic approaches within past 30 years. Molecular biology methods have been recently incorporated into the set of diagnostic methods and the importance of results obtained by molecular genetic methods for prognosis and optimal treatment is intensively studied. The treatment approaches switched from supportive care and palliative chemotherapy to new drugs that may effectively target the molecular basis of the disease as lenalidomide...

Acute myeloid leukaemia - the post and present of the treatment in the world and the Institute of Heamatology and Blood Transfusion in Prague

P. Soukup, J. Soukupová Maaloufová, J. Čermák, P. Cetkovský, M. Lukášová

Vnitr Lek 2012, 58(Suppl 2):16-19  

Despite the boom in the field of molecular genetics and genomics, acute myeloid leukemia remains an important challenge. Though patient-tailored treatment results have seen great improvement, we are still behind our and our patients expectations.

Acute lymphoblastic leukemia: past and present

C. Šálek, D. Šponerová, J. Soukupová Maaloufová

Vnitr Lek 2012, 58(Suppl 2):20-26  

Acute lymphoblastic leukemia (ALL) is a rare neoplasm in adults. Its treatment has seen dynamic developments ranging from the discovery of first compounds with cytostatic effect through to the introduction of multicomponent chemotherapy, incorporation of hematopoietic stem cell transplantation into postconsolidation therapy as well as dose intensity adjustment according to defined risk groups. The basic therapeutical principle of present treatment strategies is an individualised approach. Modern protocols build on the prognostic significance of minimal residual disease and incorporate new drugs such as tyrosine kinase inhibitors, monoclonal antibodies...

Chronic myeloid leukaemia - a crucial change to the patient prognosis after an introduction of tyrosine kinase inhibitors

H. Klamová

Vnitr Lek 2012, 58(Suppl 2):27-37  

Chronic myeloid leukaemia (CML) is an example of a disease where new knowledge in molecular biology enabled thorough understanding of the nature of the disease as well as it led to a discovery of completely new, targeted treatment principle, i.e. inhibition of tyrosine kinase Bcr-Abl, now the main therapeutic target. Introduction of tyrosine kinase inhibitors (TKI) imatinib, dasatinib, nilotinib into clinical practice meant a revolutionary change to treatment strategy with a significant effect on survival and patient quality of life. A disease with originally fatal prognosis has changed in patients diagnosed in its chronic phase to a disease with expected...

Molecular genetics in the chronic myeloid leukemia diagnostics and therapy

K. Machová Poláková, K. Zemanová, M. Součková, A. Broučková, H. Klamová

Vnitr Lek 2012, 58(Suppl 2):38-45  

This overview discusses an importance of molecular diagnostics of chronic myeloid leukemia, molecular monitoring of treatment efficacy, residual disease and resistance to therapy and the role of the National reference laboratory ÚHKT in these issues. The qualitative detection based on the multiplex reverse transcriptase PCR confirms the presence of mRNA of the fusion gene BCR-ABL in the examined sample, thus a diagnosis of chronic myeloid leukemia. Characterization of the type of BCR-ABL rearrangement is also important for the subsequent monitoring based on the quantification of BCR-ABL transcripts. The quantitative determination of BCR-ABL transcripts...

Transplantation of haematopoietic cells and its role in the treatment of haematopoiesis during the last 25 years

A. Vítek, M. Lukášová, V. Chudomel, A. Májský, J. Souček, P. Kobylka, P. Kořínková, M. Loudová, M. Dobrovolná, E. Matějková, M. Písačka, Z. Sieglová, M. Vraná, J. Hrabánek, J. Sajdová, D. Šponerová, M. Marková, V. Válková, Z. Gašová, P. Cetkovský

Vnitr Lek 2012, 58(Suppl 2):46-55  

Haematopoietic cell transplantation is a life-saving procedure in a number of haematological diseases. Development of transplantation dates back to 1970s, following discovery of the HLA system. The Institute of Haematology and Blood Transfusion in Prague had initiated its transplantation programme in 1986 and 918 haematopoietic cell transplantations were performed for various haematological diseases until 2011. Long-term survival of all patients after transplantation is around 50%, ranging from 20 to 90%, depending on the type of the disease and its stage, the type of a donor and the patient's age.

Prevention and treatment of infectious complications in haematooncology

M. Kouba, M. Lukášová

Vnitr Lek 2012, 58(Suppl 2):56-60  

Infectious complications are besides bleeding the most frequent cause of morbidity and mortality in otherwise succesful anti-leukemic therapy. Use of antimicrobial drugs and measures accompany the progress of the anti-leukemic therapy. We shortly summarize the literature on antimicrobial supportive care and the story of this field in the Institute of Hematology and Blood Transfusion, Prague. Our current diagnostic, prophylactic and therapeutic strategies are mentioned finally.

Emergency situations in hematology

M. Marková

Vnitr Lek 2012, 58(Suppl 2):61-64  

Both the development of new therapeutic strategies and intensification of the treatment of haematologic patients lead to increasing number of emergencies that must be immediately resolved. Some of these complications may be specific for this type of patients and they frequently occur at the time diagnosis and initial therapy. Although this type of treatment is usually provided in hematologic centers, the patients often need primary care immediately when complication appears. The paper should shortly outline the specific problems of these cases.

Care for patients with haemophilia and their families at The Institute of Haematology and Blood Transfusion in Prague

I. Hrachovinová, P. Salaj

Vnitr Lek 2012, 58(Suppl 2):65-69  

Beginning of the treatment of patients with hemophilia in IHBT (Institute of Hematology and Blood Transfusion) dates back to the 60th the last century. To date it was created a comprehensive care; from diagnostic to preventive and therapeutic. Diagnostic is concentrated in the NRL for hemostasis, there got all the available methodologies, protein and molecular-genetic. Therapeutic and preventive care is concentrated in the Center for Thrombosis and Hemostasis (CTH IHBT) and clinical departments of IHBT. In collaboration with orthopedic, surgical and other specialized clinics they provide care during invasive procedures. IHBT was appointed as one of...

Studies of structural and functional changes of fibrinogen

J. Štikarová, R. Kotlín, J. Suttnar, T. Riedel, J. E. Dyr

Vnitr Lek 2012, 58(Suppl 2):70-83  

At the Institute of Hematology and Blood Transfusion, we have been studying hereditary dysfibrinogenemia for more than ten years. During this period we have described more than 30 families in the Czech Republic with inherited mutations in fibrinogen. This paper provides an overview of 8 interesting cases of dysfibrinogenemia which we have characterized. Individual cases differ mainly in their clinical manifestations. The study of congenital fibrinogen disorders provides scientists and clinicians important information on structural and functional aspects of the fibrinogen molecule during various physiological processes, especially hemostasis. For roughly...

Use of label-free protein chips for proteomics of oncohematological diseases

K. Pimková, J. Suttnar, M. Bocková, J. Homola, J. E. Dyr

Vnitr Lek 2012, 58(Suppl 2):84-89  

Despite great advances in our understanding of the molecular basis of many diseases, there are still substantial gaps in our understanding of oncohematological diseases as well as in the development of effective strategies for early diagnosis and for treatment. The current interest in proteomics is growing partly due to the prospects that proteomic methods offer and hopefully overcome limitations of other approaches. At the Institute of Hematology and Blood Transfusion the proteomics of oncohematological diseases, especially myelodysplastic syndrome and chronic myeloid leukaemia, has been studied. This work gives an overview of development and importance...

Hemapheresis - the efficient therapeutic technique in clinical practice

Z. Gašová

Vnitr Lek 2012, 58(Suppl 2):91-102  

Hemapheresis technique has been used widely in the clinical practice. It enables preparing high yields of blood components or helps to remove the pathogenetic substance from the patient's circulation. In the Institute of Hematology and Blood Transfusion many types of therapeutic methods have been used. Procedures include cell depletion - with removing of the extent of leukocytes, red cell and platelets from the patient's circulation. Other methods are therapeutic plasma exchange, red blood cells exchange, immunoadsorption of IgG, extracorporeal photochemotherapy (ECP), and peripheral blood progenitor cells (PBPC) collections. In our previous studies...

Immunohaematology - the history, current knowledge and the role of The Institute of Haematology and Blood Transfusion in Prague

M. Písačka, P. Kořínková, E. Matějková, H. T. Bolcková

Vnitr Lek 2012, 58(Suppl 2):103-114  

Immunohaematology is a branch of the medical science dealing with interactions of the immune system and blood, namely with the detection of blood cells antigens and related antibodies. The discovery of immunohaematology principles in the beginning of 20th century has broken the immune barrier of the blood transfusion and enabled the progress in the transfusion medicine and in all other medical disciplines requiring the transfusion support. Routine use of immunohaematology tests serves for the assurance of compatible blood transfusions and for the diagnostics, monitoring and treatment of allo- and autoimmune cytopenias. So far more than 300 red cell...

Iron metabolism and its regulations

D. Vyoral, J. Petrák

Vnitr Lek 2012, 58(Suppl 2):115-126  

Iron is an indispensable element. As a catalytical center in many Fe metalloproteins it participates in almost all biochemical pathways. This element, however is able to initiate the generation of toxic free radicals. This review article summarizes the available knowledge on the mechanisms, regulations and disorders of iron absorption, transport and storage.